肾孤立性纤维瘤临床病理观察

目的探讨肾孤立性纤维瘤的临床病理特征、诊断和鉴别诊断，提高对该肿瘤的诊断水平。方法对1例肾孤立性纤维瘤进行光镜、免疫组化观察，并结合文献讨论。结果患者女性，47岁。临床、放射学诊断为肾盂癌。肿瘤边界清楚，切面灰白色。镜下梭形细胞呈束状、旋涡状或不规则状排列，部分与胶原纤维混杂，部分呈血管外皮瘤样结构。细胞无明显异型，核分裂象偶见。免疫组化示肿瘤细胞vimentin、CD34和皿199（＋），bel-2部分（＋），desmin、SMA、MAS、HMB45、carponin、S-100蛋白、CD117、CD31、FVⅢ、CK（AE1/AE3）、ENA、Ki-67和p53均（-）。结论肾孤立性纤维瘤是一种非常罕见的肿瘤，诊断主要依靠病理形态学及免疫组化，并应与肾的其他梭形细胞肿瘤鉴别。     

乳腺恶性孤立性纤维性肿瘤两例

本文分析并总结了北京协和医院乳腺外科诊治的2例乳腺恶性孤立性纤维性肿瘤(solitary fibrous tumor, SFT)的临床及病理特点。2例患者均以乳腺巨大肿物为主要表现, 行乳腺肿物局部扩大切除术治疗, 未清扫患侧腋窝淋巴结; 术后病理提示CD34(+)、S-100(-), 符合SFT免疫组化特点; 因肿瘤细胞存在多形性及高核分裂相, 故诊断为恶性SFT。2例术后均未行辅助治疗, 1例患者于术后6个月出现局部复发, 目前均无远处转移表现。乳腺原发恶性SFT需病理明确诊断, 乳腺肿物局部扩大切除或患侧乳腺单纯切除是主要治疗手段, 目前无明确证据表明腋窝淋巴结清扫及辅助放化疗可延长患者生存期。     

6例恶性孤立性纤维性肿瘤的临床病理特征

回顾性分析6例恶性孤立性纤维性肿瘤(malignant solitar y f ibrous tumor,MSFT)的临床病理特征,其中男5例,女1例;年龄25~69(中位数42.17)岁,分别发生在前额、膝部、大腿、锁骨上及胸腹壁,肿瘤直径1.8~24.5 cm。组织学特点:瘤细胞丰富密集,其间可见绳索样、玻璃样变胶原纤维分隔,瘤细胞呈短梭形、梭形,异型性明显,核分裂易见(≥4/10 HPF),瘤细胞呈束状、漩涡状排列,部分区域可见血管外皮瘤样结构,局灶可见出血或肿瘤性坏死。肿瘤部分区域可见浸润性边缘。免疫组织化学特点:6例均弥漫表达波形蛋白(Vimentin),STAT6,CD34,Bcl-2和CD99,1例的孕激素受体(progesterone receptor,PR)局灶阳性,6例的Ki-67为2%~60%(+)。S100,AE1/AE3,EMA,CD117,SOX10,Desmin,平滑肌肌动蛋白(smooth muscle actin,SMA),雌激素受体(estrogen receptor,ER)均阴性。MSFT的确诊主要依赖组织学的形态特征和免疫组织化学染色。形态学非常温和良善的SFT也可能有侵袭性生物学行为,临床上应尽早完整切除肿物并对孤立性纤维性肿瘤(solitar y fibrous tumor,SFT)长期随访,注意恶变的可能性。手术切除的完整性以及必要的放/化疗、靶向药物治疗等对预后非常重要。     

A Solitary Fibrous Tumour of the Eyelid

Objective

To report a case of palpebral solitary fibrous tumour (SFT).

Clinical Presentation and Intervention

An elderly man presented with a slow-growing painless mass in the lower conjunctival fornix in the left eye. The lesion was excised and it measured 0.9 cm in maximum diameter. The microscopic features were characteristic of a benign SFT, with immunohistochemical reactivity for vimentin, CD34 and Bcl-2 protein. Nuclear staining for progesterone receptor was also observed.

Conclusion

This tumour displayed a benign course, with no recurrence after excision. CD34 immunohistochemistry proved to be a useful adjunct to the microscopic diagnosis.Key Words: Solitary fibrous tumour, Eyelid, CD34, Progesterone receptor, Bcl-2     

鞍区孤立性纤维性肿瘤临床病理观察

目的 探讨鞍区孤立性纤维性肿瘤的临床病理学特点及诊断要点.方法 对1例鞍区孤立性纤维性肿瘤进行临床资料、影像学、病理形态学观察及免疫组化检测,并结合文献对其诊断及鉴别诊断进行探讨.结果 患者女性43岁.MIR提示鞍区占位,肿瘤向鞍上及海绵窦侵犯.镜下可见梭形细胞结构,局部细胞密度较高,可见红染无细胞区及退变,核分裂平均＜2个／10HPF,细胞呈编织状排列.免疫组化:CD34、CD99、bc1-2和vimentin (+),S-100和EMA(-).结论 发生于鞍区的孤立性纤维性肿瘤罕见.由于其发病部位特殊,需将临床资料、影像学资料与病理学及免疫组化特点相结合进行诊断,并注意与垂体细胞瘤、神经鞘瘤等鉴别.     

Corosolic acid inhibits tumour growth without compromising associating liver partition and portal vein ligation-induced liver regeneration in rats

BackgroundAssociating liver partition and portal vein ligation (ALPPS) technique is a promising strategy for unresectable primary liver tumours without sufficient future liver remnants (FLRs).ObjectiveOur study explored the effect of corosolic acid (CA) on inhibiting tumour growth without compromising ALPPS-induced liver regeneration.MethodsThe ALPPS procedure was performed in Sprague–Dawley rats with orthotopic liver cancer. Blood, tumour, and FLR samples were collected, and the effect of CA on the inhibition of tumour progression and ALPPS-induced liver regeneration, and its possible mechanism, were investigated.ResultsThe tumour weight in the implantation/ALPPS group was higher than in the implantation without ALPPS group (p < .05), and the tumour weight in the implantation/ALPPS/CA group was lower than in the implantation/ALPPS group (p < .05). On postoperative day 15, the hepatic regeneration rate, and the expression of Ki67+ hepatocytes in the FLRs had increased significantly in the group that underwent ALPPS. The number of cluster of differentiation (CD) 86+ macrophages markedly increased in the FLRs and in the tumours of groups that underwent the ALPPS procedure. Additionally, the number of CD206+ macrophages was higher than the number of CD86+ macrophages in the tumours of the implantation and the implantation/ALPPS groups (p < .01, respectively); however, the opposite results were observed in the CA groups. The administration of CA downregulated the expression of transforming growth factor-beta (TGF-β), CD31, and programmed cell death protein 1 (PD-1) but increased the number of CD8+ lymphocytes in tumours.ConclusionCorosolic acid inhibits tumour growth without compromising ALPPS-induced liver regeneration. This result may be attributed to the CA-induced downregulation of PD-1 and TGF-β expression and the increased CD8+ lymphocyte infiltration in tumour tissue associated with the suppression of M2 macrophage polarisation.

Key Messages

• This study aimed to investigate the effect of CA on ALPPS-induced liver regeneration and hepatic tumour progression after ALPPS-induced liver regeneration.
• Corosolic acid inhibits tumour growth without compromising ALPPS-induced liver regeneration. This result may be attributed to the CA-induced downregulation of PD-1 and TGF-β expression and the increased CD8+ lymphocyte infiltration in tumour tissue associated with the suppression of M2 macrophage polarisation.

     

颅内血管周细胞瘤MRI表现及病理分析

目的 分析颅内血管周细胞瘤(HPC)的MRI表现,并与病理结果相对照.方法 收集经病理及免疫组化检查确诊的7例HPC患者资料,回顾性分析其临床表现、病理特征及MRI表现.结果 7例HPC中,6例病变位于小脑幕上,1例跨小脑幕上下生长;其中2例小脑幕上病例累及邻近骨质.颅内HPC呈等及稍长T1稍长T2信号,肿块内可见囊变坏死、出血及流空血管,周围见轻度水肿;增强扫描后肿块实质性部分明显强化,2例可见“脑膜尾征”,2例累及邻近骨质,1例侵及大脑大静脉及大脑内静脉.免疫组化结果显示7例均见CD34、波形蛋白及CD99表达,上皮细胞膜抗原、细胞角蛋白、胶质纤维酸性蛋白及S-100蛋白均未见表达;对6例进行Bcl-2标记均见表达.对4例进行增殖细胞核抗原Ki-67标记,其中3例为5％～8％表达,1例为10％～15％表达.对3例进行Syn染色,结果均为阴性.结论 MRI对诊断颅内HPC有重要参考价值,但确诊仍依赖于病理和免疫组织化学检查.     

Pediatric home chemotherapy: infusing "quality of life".

A pediatric home chemotherapy program is described that has operated as part of the pediatric oncology services at the Children's Hospital at Montefiore since 2004. Currently the chemotherapy regimens include high-dose methotrexate; 5-day high-dose ifosfamide and etoposide; cyclophosphamide, doxorubicin, and vincristine; ifosfamide, carboplatin, and etoposide; and cisplatin and doxorubicin. The pediatric hematology/oncology program provides care to children with blood disorders and cancer of Bronx, NY, and surrounding areas. By providing patients receiving chemotherapy treatment with this special type of home infusion capability, the child and his or her family are able to spend more time at home and have less disruption in their family schedule.     

Immunohistochemical analysis and biological behaviour of gastric glomus tumours: a case report and review of the literature

Gastric glomus tumours are rare and clinically recognized as benign. Nevertheless, some show biological behaviour similar to that of malignant lesions. During the last 40 years, we have encountered only one gastric glomus tumour. Analysis of frozen sections of this tumour suggested a mesenchymal tumour with malignant potential. Three mitoses per 50 high-power fields, with no cytological abnormalities, were observed. Tumour cells were positive for α-smooth muscle actin, vimentin and actin but negative for CD117, S-100 protein, creatine kinase, desmin, CD68, collagen type IV, CD34 and p53. The post-operative period was uneventful. During 37 months' follow-up, no recurrence or metastasis was detected and a benign course was considered likely. Literature on the immunohistochemistry and biological behaviour of gastric glomus tumours was also reviewed. Immunohistochemical studies are helpful in the differential diagnosis of gastric glomus tumours: although most are benign, malignancy cannot be excluded. Thus, long-term follow-up of the patient is necessary.     

Prophylactic treatment of known ifosfamide-induced encephalopathy for chemotherapy with high-dose ifosfamide?

We report about a case of advanced Ewing sarcoma in a 30-year-old woman. Initial treatment was started according to the Euro-Ewing 99 protocol. During the initial therapy, an ifosfamide-induced encephalopathy occurred as status epilepticus. Because of cerebral toxicity, the following chemotherapies went without ifosfamide. During final radiotherapy multiple lung metastasis were diagnosed. After two cycles of chemotherapy with cyclophosphamide and topotecan (no response), left thoracotomy, and palliative pneumectomy, the patient was transferred to our ward for further treatment. Undergoing two cycles of chemotherapy with ifosfamide 4 g/m² intravenously for 3 consecutive days followed by high-dose chemotherapy (HDCT) according to the ICE-regimen (ifosfamide 2 g/m², carboplatin 200 mg/m², and etoposide 2×100 mg/m² intravenously for 6 consecutive days), and peripheral blood stem cell transplantation (PBSCT), complete remission was achieved. Under preventive therapy with methylene blue, thiamin, and glucose 5% infusions, no encephalopathy occurred.     

孤立性纤维性肿瘤超声造影和病理学诊断特征分析

目的探讨孤立性纤维性肿瘤超声造影和病理学的诊断特征。 方法回顾性分析2015年1月至2019年12月青岛市市立医院经手术与病理证实的6例孤立性纤维性肿瘤患者的超声造影和病理学资料,总结其诊断学特征。 结果6例患者中,男性3例,女性3例,年龄32～65岁,1例位于腹壁,1例位于足背,1例位于眼眶,2例位于胸壁,1例位于乳腺。6例孤立性纤维性肿瘤二维超声表现为边界清楚或不清、边缘光滑的低回声或中等或混合回声肿块,4例呈椭圆形,2例呈圆形;2例回声欠均匀伴液化,4例回声较均匀;4例可见丰富的分支状血流信号,血管阻力较小。超声造影均呈动脉期均匀性或不均匀性高增强,2例病灶增强范围较常规超声增大,4例病灶增强范围较常规超声无明显增大,延迟期无明显廓清,呈略高增强。6例均经手术切除,4例可见完整包膜,2例与周围组织界限不清;4例有丰富的血管供应,2例可见坏死液化区,4例质韧,2例质地中等。术后常规苏木精-伊红(HE)染色示间叶组织来源肿瘤,瘤细胞呈梭型,围绕血管生长,呈旋涡状或索状,细胞聚集区与稀疏区交替分布;肿瘤细胞分布不均,间质内见粗细不等红染的胶原纤维及丰富树枝状薄壁血管;4例核有轻度异型性,核分裂象少见,2例核有中度异型性,核分裂象多见。免疫组化结果示：CD34阳性(4/6),B淋巴细胞瘤2(Bcl-2)阳性(5/6),波形蛋白(Vimentin)阳性(5/6),CD99阳性(6/6),平滑肌肌动蛋白(SMA)阳性(4/6),S100阴性(6/6),符合孤立性纤维性肿瘤的诊断。 结论孤立性纤维性肿瘤的超声造影和病理学具有一定的特征性,有助于其诊断。     

肾原发性孤立性纤维性肿瘤临床病理观察

目的 探讨原发于肾的孤立性纤维性肿瘤的临床病理特点及鉴别诊断要点.方法 复习2例肾孤立性纤维性肿瘤患者的临床资料,并对其进行组织学观察和免疫组化标记.结果 2例肾原发性孤立性纤维性肿瘤患者均为女性,年龄分别为33岁和63岁.临床主要表现为肾区钝痛,无尿频、尿急、尿痛,无血尿.CT检查示肾占位.组织学特征为瘤组织呈束状、波纹状排列,瘤细胞呈梭形,局部富于瘤细胞,间质血管丰富,部分呈血管瘤样或血管外皮瘤样结构,有明显的束状胶原.免疫表型：瘤细胞CD34、CD99和bcl-2（＋）,SMA局灶性（＋）;而HMB45和CD10（-）.结论 孤立性纤维性肿瘤具有低度恶性潜能,发生在肾极少见,以手术治疗为主,预后较好.确诊主要依靠组织病理学,并辅以免疫组化标记.     

外周性原始神经外胚层瘤15例临床分析

目的：分析外周性原始神经外胚层瘤（peripheralprimitiveneuroectodermaltumors,pPNET）的特点、治疗及预后。方法：对2006年6月2011年5月的15例pPNET患者的临床资料进行分析。其中男性7例,女性8例;中位年龄23岁（11～55岁）。原发部位：骨6例,软组织4例,胸壁1例（Askin瘤）,纵隔1例,后腹膜1例,肾脏1例,头皮1例。所有病例均采用手术或放疗以及全身化疗。化疗方案有3药联合[异环磷酰胺／环磷酰胺＋蒽环类药物（阿霉素／表阿霉素）＋顺铂、异环磷酰胺＋阿霉素＋氮烯米胺、异环磷酰胺／环磷酰胺＋阿霉素＋长春新碱]或2药联合（异环磷酰胺＋依托泊甙、异环磷酰胺＋阿霉素）。结果：5例辅助化疗的患者均无复发（随访期6～42个月,中位随访时间21个月）。10例姑息化疗的患者中,完全缓解（completeresponse,CR）1例、部分缓解（partialresponse,PR）3例、疾病稳定（stabledisease,SD）3例、疾病进展（progressivedisease,PD）3例（其中2例病死）,总有效率（CR＋PR＋SD）达70％;3例PD患者疾病进展时间（timetoprogression,TTP）分别是2．4、7、8个月。所有病例中位生存期尚未达到。结论：pPNET是一种高度恶性和异质性的肿瘤,预后差,主要依靠病理学诊断;采用合理的综合治疗方案可延长患者生存期,VAC／IE交替方案治疗pPNET是有前景且安全性较好的化疗方案。     

Macrophages expressing TREM-1 are involved in the progression of HPV16-related oropharyngeal squamous cell carcinoma

IntroductionMany types of research have been performed to improve the diagnosis, therapy, and prognosis of oropharyngeal carcinomas (OP-SCCs). Since they arise in lymphoid-rich areas and intense lymphocytic infiltration has been related to a better prognosis, a TREM-1 putative function in tumour progression and survival has been hypothesized.Materials and methodsTwenty-seven human papillomavirus (HPV) 16⁺ OP-SCC specimens have been analyzed to relate TREM-1 expression with histiocytic and lymphocytic markers, HPV presence and patients’ outcome.ResultsNo differences have been shown between intratumoral and stromal CD4⁺ cells, while intratumoral CD8⁺ lymphocytes are higher with respect to the tumour stroma (p = .0005). CD68⁺ cells are more than CD35⁺ and TREM-1⁺; their presence is related to CD35^± and TREM-1^± histiocytes (p = .005 and .026, respectively). Intratumoral CD4⁺ lymphocytes are higher in p16⁺ cases (11/27) than in p16⁻ (p = .042); moreover, p16 positivity correlates to a better survival (p = .034). CD4⁺, CD8⁺ and CD35⁺ cells have no impact on survival, while CD68 expression heavily influences progression and bad outcome (p = .037). TREM-1 positivity also leads to worst overall survival (p = .001): peritumoral expression and death-cause relationship are always significant, particularly when the cause is OP-SCC (p = .000).ConclusionWhile p16 shows to better stratify HPV16⁺ patients’ outcome, TREM-1⁺ macrophages suggest their key importance in HPV-related OP-SCCs progression.

KEY MESSAGES

• TREM-1 positivity correlates to the worst overall survival of HPV16-positive OPSCCs-affected patients.
• p16-positive HPV16 related OPSCCs patients have a better prognosis with respect to p16-negative ones.

     

腹膜后炎症性恶性纤维组织细胞瘤与Rosai-Dorfman病的鉴别诊断

目的 报道1 例S-100(+)的腹膜后炎症性恶性纤维组织细胞瘤并探讨其与Rosai-Dorfman 病的鉴别.方法 进行临床病理和免疫组化分析, 结合文献讨论其诊断与鉴别.结果 患者女性, 88岁. 腹痛1个月.CT检查示腹膜后巨大肿块,广泛浸润邻近器官.病理检查示肿瘤由不同分化程度的组织细胞、泡沫细胞、纤维细胞及多种炎性细胞构成.肿瘤性组织细胞及纤维母细胞形成明显的车辐状结构, 组织细胞无吞噬淋巴细胞.免疫组化:组织细胞S-100、CD68和vimentin(+);而EMA、CD21、CD35、ALK-1、CD1α和desmin(-).结论 炎症性恶性纤维组织细胞瘤表达S-100蛋白相当罕见,与Rosai-Dorfman 病鉴别诊断需要结合临床特点、组织病理学和免疫组化综合分析.形态学上炎症性恶性纤维组织细胞瘤中的组织细胞比Rosai-Dorfman病中的组织细胞小、 形态多样、中度异型性、出现病理性核分裂象,无吞噬淋巴细胞现象和背景中有多量中性粒细胞是与Rosai-Dorfman病鉴别诊断的要点.     

附睾横纹肌肉瘤的临床病理学特征并文献复习

目的探讨附睾横纹肌肉瘤的临床病理学特征。 方法选择2011年1月至2019年12月江苏省沭阳人民医院泌尿外科收治的3例附睾横纹肌肉瘤患者，应用常规病理检查和免疫组织化学方法，分析附睾横纹肌肉瘤的临床病理学特征。 结果3例附睾横纹肌肉瘤患者的年龄分别为10岁、23岁和25岁，病程分别为1个月、2个月和4个月。左侧附睾1例，右侧附睾2例，均为单发性病灶，临床以阴囊肿块并进行性增大为主要症状。B超和磁共振成像(MRI)检查均发现附睾占位性病变，术后病理诊断为附睾横纹肌肉瘤，其中2例为腺泡状横纹肌肉瘤，1例为胚胎性横纹肌肉瘤。病理组织学检查显示肿瘤由原始间叶细胞和不同分化的横纹肌母细胞组成，免疫组织化学显示肌细胞生成素(myogenin)、肌调节蛋白(MyoD1)、波形蛋白(vimentin)和结蛋白(desmin)阳性，神经元特异性稀醇化酶(NSE)、S-100蛋白、肌动蛋白(actin)、平滑肌肌动蛋白(SMA)、内皮细胞标记物(CD34)、尤文氏肉瘤标记物(CD99)和广谱细胞角蛋白(CKpan)阴性。 结论附睾横纹肌肉瘤是一种比较少见的恶性肿瘤，早期诊断和规范治疗可获得较好的预后。     

多形性黄色瘤型星形细胞瘤及巨细胞胶质母细胞瘤的临床病理分析

目的探讨多形性黄色瘤型星形细胞瘤(PXA)及巨细胞胶质母细胞瘤(GCG)的临床病理特点、诊断及鉴别诊断。方法对4例PXA及6例GCG进行临床病理学观察,免疫组化分析。结果PXA患者组织学特征:肿瘤由巨怪瘤细胞、梭形细胞和泡沫样瘤细胞组成,有丰富的网状纤维及淋巴细胞浸润,坏死和核分裂像少见。免疫组织化学染色GFAP、Vim、S-100阳性,呈弥漫性表达,CD34阳性率75%。GCG患者组织学特征:瘤细胞多形性,以巨怪形瘤巨细胞为主,核分裂像和出血坏死多见,网状纤维沿血管周围分布,有淋巴细胞浸润。瘤细胞弥漫性表达Vim、S-100,GFAP灶性阳性,CD34阳性率17%。结论PXA及GCG临床及病理组织形态非常相似,两者鉴别诊断的要点在于有无泡沫样瘤巨细胞、核分裂像、坏死以及CD34、GFAP的表达不同。     

The correlation between shoulder pathologies and sleep disorders

ObjectiveTo assess and characterize the correlation between shoulder pathologies and sleep disturbances.MethodsParticipants enrolled into this case–control study were divided into two groups: patients with an established clinical diagnosis of active shoulder pathology (study group), and patients without any shoulder pathology (control group). All patients completed the Insomnia Severity Index (ISI) questionnaire, in addition to questions related to participant demographics, health status, medication, and other known insomnia risk factors.ResultsA total of 98 patients were included (46 in the study group and 52 controls). Mean ISI score was significantly higher (indicating more severe insomnia) in the study group versus control group (t[96] = –9.67), even after correcting for confounders (t[53.1] = –8.61). Additionally, in patients with shoulder pathology, those with comorbidities experienced more sleep disturbances than those without comorbidities (β = 0.36). Lastly, the shoulder pathology group was at a higher risk of having sleep disturbances compared with controls (relative risk 4.86, 95% confidence interval 2.24, 10.55).ConclusionsSleep disturbances are more common among patients with shoulder pathologies. Comorbidities and a shorter duration of pathology may predict more severe sleep disturbances.     

肾脏巨大黏液样小管状梭形细胞癌一例并文献复习

目的分析并探讨肾脏黏液样小管状和梭形细胞癌的临床、影像及病理学特点。方法对1例肾脏巨大黏液样小管状和梭形细胞癌患者的临床资料进行回顾性报道并相关文献复习分析,其早期发现由6 cm发展5年至18 cm巨大肿块出现腹腔挤压情况方行治疗,640排CT检查与其他肾脏肿瘤无特异性区别,行肾癌根治性切除术,术后病理确诊。结果病理检查示:镜下见肿瘤组织由狭长的小管组成,小管间见黏液间质,肿瘤细胞呈卵圆形及杆状,中度异性,间质内见多灶性浆细胞浸润,伴大片坏死。免疫组化:CD10(-),VIM(+),EMA(+),低分子量CK(-),CD15(-),Ki-67<5%肿瘤细胞,CK(-),CR(-),MC(-),CD34(-),CD31(-),S-100(-),诊断为肾黏液样小管状梭形细胞癌。术后恢复良好,随访3个月复查CT无复发,日常中度活动无不适反应,目前继续追踪随访中。结论肾脏黏液样小管状梭形细胞癌是一种罕见的低度恶性肾上皮肿瘤,术前诊断无特异性,最终依赖术后病理确诊,治疗仍以根治性切除术为主,预后相对较好。